Granulomatosis with polyangiitis presenting with pachymeningitis and persistent headache

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a systemic inflammatory disease, primarily involving the upper and lower respiratory tract and kidneys. It is a relatively uncommon condition, characterized by necrotizing granulomatous vasculitis of small- and medium-sized vessels and the presence of anti-neutrophil cytoplasmic antibodies in the serum as defined in the Chapel Hill Consensus Conference 2012. GPA presents a wide spectrum of manifestations and remains one of the most challenging diagnostic dilemmas in clinical medicine. From common respiratory and neurological symptoms to infrequent cardiac complications, this fatal systemic illness is difficult to distinguish from infectious etiologies, and it is often mistaken for an isolated complaint. We report a case of granulomatosis with polyangiitis in a patient with persistent frontal headache, for a very long time considered as migraine versus tension-type headache.